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Coenzyme Q supplementation in pulmonary arterial hypertension

Significance Statement

Pulmonary arterial hypertension is a disease that affects the blood vessels of the lungs, causing high pressures that lead to right-sided heart failure.  Abnormalities of the mitochondria, the powerhouse of the cells of the body, are present in the blood vessels of patients with pulmonary arterial hypertension (PAH). The mitochondria produce energy, but also are required for the production of heme that is used in hemoglobin in red blood cells.  Coenzyme Q (CoQ10) is a vitamin cofactor, that is essential for mitochondria functions. Coenzyme Q  has been shown to improve heart function in cardiac failure patients through effects on the mitochondria, so we hypothesized that Coenzyme Q  might improve mitochondrial functions in energy and heme production and benefit PAH patients.  We conducted a prospective study where 8 PAH patients and 7 healthy controls were supplemented with oral Coenzyme Q  (100mg three times a day) for 12 weeks. Coenzyme Q  levels were similar among PAH and controls at baseline.  However, PAH patients had higher Coenzyme Q  levels than controls after taking Coenzyme Q  supplementation. Many studies have identified abnormalities in iron metabolism and red blood cells in pulmonary hypertension patients. Here, we found similar abnormalities in patients at baseline, but also found that Coenzyme Q  improved the hemoglobin content of red blood cells  in the PAH patients.  These changes were not seen in healthy subjects, in fact hemoglobin decreased slightly in healthy controls. Coenzyme Q improved echocardiographic right heart function in PAH subjects, but exercise and other markers of heart failure, such as serum brain naturetic peptide (BNP), did not improve over the course of the 3 month study.  Equally important, Coenzyme Q  was well tolerated in this study with minimal side effects.  The decrease in immature red blood cells, improvements in heart function by echocardiogram, and increased red cell hemoglobin at 12 weeks suggest that trials of longer duration, and/or higher doses of Coenzyme Q .

Coenzyme Q supplementation in pulmonary arterial hypertension. Global Medical Discovery

 

 

 

 

 

 

 

 

 

 

 

 

 

 

Journal Reference

Sharp J1, Farha S2, Park MM3, Comhair SA4, Lundgrin EL5, Tang WH6, Bongard RD7, Merker MP8, Erzurum SC9. Redox Biol. 2014 ;2:884-91.

Show Affiliations

1Respiratory Institute, Cleveland Clinic, 9500 Euclid Avenue, Cleveland, OH 44195, United States ; Department of Pathobiology, Lerner Research Institute, Cleveland Clinic, 9500 Euclid Avenue, Cleveland, OH 44195, United States ; Heart and Vascular Institute, Cleveland Clinic, 9500 Euclid Avenue, Cleveland, OH 44195, United States.

2Respiratory Institute, Cleveland Clinic, 9500 Euclid Avenue, Cleveland, OH 44195, United States ; Department of Pathobiology, Lerner Research Institute, Cleveland Clinic, 9500 Euclid Avenue, Cleveland, OH 44195, United States.

3Respiratory Institute, Cleveland Clinic, 9500 Euclid Avenue, Cleveland, OH 44195, United States ; Heart and Vascular Institute, Cleveland Clinic, 9500 Euclid Avenue, Cleveland, OH 44195, United States.

4Respiratory Institute, Cleveland Clinic, 9500 Euclid Avenue, Cleveland, OH 44195, United States ; Cleveland Clinic Lerner College of Medicine, Cleveland Clinic, 9500 Euclid Avenue, Cleveland, OH 44195, United States.

5Cleveland Clinic Lerner College of Medicine, Cleveland Clinic, 9500 Euclid Avenue, Cleveland, OH 44195, United States.

6Heart and Vascular Institute, Cleveland Clinic, 9500 Euclid Avenue, Cleveland, OH 44195, United States ; Cleveland Clinic Lerner College of Medicine, Cleveland Clinic, 9500 Euclid Avenue, Cleveland, OH 44195, United States.

7Department of Medicine, Medical College of Wisconsin, 8701 Watertown Plank Road, Milwaukee, WI 53226, United States.

8Department of Anesthesiology, Medical College of Wisconsin, 8701 Watertown Plank Road, Milwaukee, WI 53226, United States ; Department of Pharmacology and Toxicology, Medical College of Wisconsin, 8701 Watertown Plank Road, Milwaukee, 53226, United States ; Zablocki VAMC, 5000 W National Ave., Milwaukee, WI 53295, United States.

9Respiratory Institute, Cleveland Clinic, 9500 Euclid Avenue, Cleveland, OH 44195, United States ; Department of Pathobiology, Lerner Research Institute, Cleveland Clinic, 9500 Euclid Avenue, Cleveland, OH 44195, United States ; Cleveland Clinic Lerner College of Medicine, Cleveland Clinic, 9500 Euclid Avenue, Cleveland, OH 44195, United States.

 

ABSTRACT

Mitochondrial dysfunction is a fundamental abnormality in the vascular endothelium and smooth muscle of patients with pulmonary arterial hypertension (PAH). Because coenzyme Q (CoQ) is essential for mitochondrial function and efficient oxygen utilization as the electron carrier in the inner mitochondrial membrane, we hypothesized that Coenzyme Q  would improve mitochondrial function and benefit PAH patients. To test this, oxidized and reduced levels of Coenzyme Q , cardiac function by echocardiogram, mitochondrial functions of heme synthesis and cellular metabolism were evaluated in PAH patients (N=8) in comparison to healthy controls (N=7), at baseline and after 12 weeks oral Coenzyme Q  supplementation. Coenzyme Q  levels were similar among PAH and control individuals, and increased in all subjects with Coenzyme Q  supplementation. PAH patients had higher Coenzyme Q  levels than controls with supplementation, and a tendency to a higher reduced-to-oxidized Coenzyme Q  ratio. Cardiac parameters improved with CoQ supplementation, although 6-minute walk distances and BNP levels did not significantly change. Consistent with improved mitochondrial synthetic function, hemoglobin increased and red cell distribution width (RDW) decreased in PAH patients with Coenzyme Q , while hemoglobin declined slightly and RDW did not change in healthy controls. In contrast, metabolic and redox parameters, including lactate, pyruvate and reduced or oxidized gluthathione, did not change in PAH patients with Coenzyme Q . In summary, Coenzyme Q  improved hemoglobin and red cell maturation in PAH, but longer studies and/or higher doses with a randomized placebo-controlled controlled design are necessary to evaluate the clinical benefit of this simple nutritional supplement.

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